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Case Report

Autoimmune Hepatitis SLE Overlap Syndrome

Jency Maria Koshy*, Mary John**
Abstract
Autoimmune hepatitis also known as Lupoid hepatitis is an autoimmune liver disease characterized by the
presence of autoantibodies including antinuclear antibodies (ANA) and hyper gammaglobulinemia. SLE can be
associated with hepatitis, which is referred to as lupus hepatitis. It is important to distinguish these two entities,
as the course of the disease is different in both. It has also been noted that these two entities can co-exist when it
is referred to as Autoimmune hepatitis-SLE overlap syndrome. We are reporting a case of Autoimmune hepatitisSLE overlap syndrome in a 30 years old lady.

Introduction

ifferentiating the hepatic involvement in Systemic lupus

Erythematosis (SLE) and Autoimmune Hepatitis (AIH) has
been challenging, as there are similarities in the clinical features
and the biochemical parameters in both these entities. AIH is
also known as Lupoid hepatitis, which is an autoimmune liver
disease, caused by the presence of autoantibodies, including
antinuclear antibodies (ANA) and hypergammaglobulinemia.
SLE can also have accompanying liver disease referred to
as lupus hepatitis though it is rare. We are reporting a case of
Autoimmune hepatitis- SLE overlap syndrome. This patient
fulfilled the American College of Rheumatology (ACR) criteria
for SLE and was proved to have AIH on liver biopsy.

Case Report
30 years old lady presented to our hospital with yellowish
discoloration of sclera and abdominal distention for 1month.
She was initiated on anti tubercular treatment for tuberculous
lymphadenitis, 10 months prior to admission. She discontinued
the medicines after 4 months as she developed jaundice following
which her jaundice subsided. She also gave history of multiple
blood transfusions in the past for anaemia. There was no history
of obvious blood loss.
Clinical examination revealed icterus, pallor, clubbing,
cervical lymphadenopathy and ascitis. There were no peripheral
signs of liver cell failure. Probable diagnoses considered on her
were Disseminated tuberculosis/ autoimmune hepatitis.
Investigations revealed anaemia (Hb 7g%), leucopoenia with
lymphopenia (TLC 3700/mm3, ALC 980/mm3), deranged PT
and PTTK, direct hyperbilirubinemia and raised transaminases.
Ascitic fluid examination was transudative. USG Abdomen
showed liver cirrhosis with portal hypertension and ascitis.
We further evaluated the patient for the various causes of liver
cirrhosis.HbsAg and HCV Ag were not detected in serum.
HEVAg was positive. KF rings were absent on slit lamp
examinaton.
Prominent esophageal vein was noted on endoscopy. CT
abdomen revealed nodular liver, dilated portal vein, multiple
lymph nodes measuring 21x 12 mm in the mesentry, portal and
aortocaval areas, ascitis and B/L pleural effusion.
Cervical lymph node biopsy and bone marrow aspiration
were done considering a possibility of disseminated tuberculosis
or lymphoma. Lymph node biopsy was reported as necrotizing
lymphadenitis. Bone Marrow examination revealed plasmacytosis
of 10-20 % with normal cell lines.
*
Assistant Professor, **Professor and Head , Department of Medicine,
Christian Medical College and Hospital, Ludhiana 141008, Punjab
Received: 13.10.2011; Accepted: 29.07.2011

Serum protein electrophoresis revealed M band in g region.

IgG was1100 U/ml (650-1500), IgA was 488U/ml (50-450) and
IgM was 260U/ml(24-332). ANA and AntiDsDNA were strongly
positive [ANA 42.6 U/ml(0-1.4 ), AntiDsDNA 213 U/ml (0-40)]
with decreased C3 and C4.
A diagnosis of SLE was made as she fulfilled ACR criteria.
(Presence of ANA, AntiDsDNA, lymphopenia and serositis).
Necrotizing lymphadenitis was attributed to SLE.However her
advanced liver cell failure could not be explained by SLE alone.
Hence we considered a possibility of Autoimmune Hepatitis
SLE overlap syndrome.
Meanwhile patients general condition worsened and she
progressed to grade 4 encephalopathy. She was pulsed with
methylprednisolone followed by oral prednisolone. However
her general condition kept worsening and she succumbed to
her illness eventually.
Postmortem liver biopsy was done which revealed large areas
of necrosis and fibrosis replacing the normal lobular architecture.
Liver cells were cord like and regenerative rosettes were present
at other places.Macrovesicular and micro vesicular steatosis
with intense lymphoplasmacytic infiltrates was present in the
fibrotic area . These findings were consistent with autoimmune
hepatitis with submassive necrosis. So the final diagnosis was
confirmed as Autoimmune Hepatitis- SLE overlap syndrome.

Discussion
Autoimmune Hepatitis (AIH) also known as Lupoid hepatitis
is an autoimmune liver disease caused by the presence of
auto antibodies, including antinuclear antibodies (ANA) and
hypergammaglobulinemia. Systemic Lupus Erythematosis
(SLE), which is an autoimmune disorder affecting multiple
organs can have accompanying liver disease, which is also
referred to as lupus hepatitis.
The difference between the hepatic involvement in SLE and
AIH has not been clearly defined due to similarities in the clinical
and biochemical features. Liver involvement in patients with SLE
is well documented but is considered rare. It has been suggested
that patients with AIH may be at an increased risk of developing
systemic connective tissue diseases. Conversely, patients with
systemic connective tissue disease may be at an increased risk
of AIH. Therefore it is important to distinguish AIH from SLE
since complications are different in the two conditions.1 SLE
may result in end stage renal disease while AIH may lead to
end stage liver disease.
Even though hepatic lesions due to pathogenic process of SLE
have been thought to be rare; recent studies have indicated that
liver involvement in patients with SLE is of a more significant
importance than had been thought.2 The difference between AIH
and hepatic lesions in SLE has long been an indistinct issue. Oka

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reported that 3% of patients with AIH satisfied the ACR criteria

for SLE.4 Matsumoto etal, who surveyed 1,468 Japanese patients
with SLE reported that only 1.1 % of them had liver cirrhosis.

Corticosteroid therapy is the mainstay of therapy.

Immunosuppresants like Azathioprine mycophenolate mofetil,
cyclosporine and tacrolimus also have shown promising results.6

AIH-SLE overlap syndrome is considered when patients

fulfill the ACR criteria for SLE and International Autoimmune
Hepatitis group scoring for AIH. Although AIH and SLE
are considered two different entity both have features of an
autoimmune disease with presence of various antibodies.
However histopathologically they are quite distinct. Periportal
piecemeal necrosis associated with lobular activity, rosetting
of liver cells or dense lymphoid infiltrates is prominent in AIH
,while in SLE, inflammation is usually lobular and occasionally
periportal with paucity of lymphoid infiltrates.

Patients presenting with AIH-SLE overlap impose diagnostic

and therapeutic dilemma on the physician. It is important to
distinguish SLE associated hepatitis (lupus hepatitis) from AIH
associated with SLE since the course of the disease is different
in both the entities.

Our patient fulfilled the ACR criteria for SLE .Her scoring as
per international Autoimmune Hepatitis group was 10.Her lower
score was attributed to unavailability of other auto antibodies
and HLA typing. She also had Hepatitis E co infection; which
also attributed to negative scoring. However the hepatotrophic
viruses are known triggers for AIH. In our patient Hepatitis E
virus infection would have been the trigger. However rosetting of
liver cells and dense lymphoplasmacytic infiltrate in our patient
was consistent with AIH. The generalized lymphadenopathy in
our patient involving cervical, mesenteric, portal and aortocaval
lymph nodes were attributed to SLE, as the biopsy was consistent
with necrotizing lymphadenitis.The stormy course with liver cell
failure was quite characteristic of AIH.
Antiribosomal P antibody is a useful marker to differentiate
SLE associated hepatitis from AIH. Anti Sm antibodies is highly
specific though relatively insensitive to SLE

Conclusion

Most of the biochemical parameters are inconclusive in

differentiating the two entities. However Liver biopsy is
diagnostic.

References
1.

Usta Y, Gurakan F, Akcoren Z, etal. An overlap syndrome involving

autoimmune hepatitis and systemic lupus Erythematosis in
childhood. World J Gastroenterol 2007;13: 2764-7.

2. Runyon BA, LaBrecque DR, Anuras S.The spectrum of liver disease

in systemic lupus Erythematosis. Report of 33 histologically- proved
cases and review of the literature. Am J Med 1980;69:187-94.
3. Tojo J, Ohira H, Abe K, etal. Autoimmune hepatitis accompanied
by systemic lupus Erythematosis. Intern Med 2004;43:258-62.
4.

Oka H.The survey of autoimmune hepatitis in Japan. Annual Report

of the Study Group on Severe Hepatitis. Japanese Ministry of Health
and Welfare 1988;235-41.

5.

Matsumoto T, YoshimineT, Shimouchi K, etal The liver in systemic

lupus Erythematosis: pathologic analysis of 52 cases and review of
Japanese Autopsy Registry data. Hum Pathol 1992;23:1151-1158.

6.

Czaja AJ. Treatment of autoimmune hepatitis. Semin Liver Dis

2002;22:365-378.